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Biomedical subjects

L Wessel

Publications and source records attributed to L Wessel.

At least 19 recordsLinked to original sources

Topographic peculiarities of the submucous plexus in the human anorectum--consequences for histopathologic evaluation of rectal biopsies.

Chronic colorectal motility disorders are commonly encountered in the pediatric population. While most cases can be managed successfully by conservative therapy, a subgroup of patients suffers from severe constipation and requires further diagnostic procedures to identify the underlying pathologies, such as aganglionosis, hypoganglionosis or intestinal neuronal dysplasia (IND). The present study provides reference data about the quantitative distribution of nerve cells and ganglia within the submucosal plexus of the human anorectum from healthy subjects. Anorectal specimens (n = 15) obtained postmortem were divided into 6 segments beginning from the dentate line (S1 = 0-2 cm, S 2 = 2-4 cm, S3 = 4-6 cm, S4 = 6-8 cm, S5 = 8-10 cm, S6 = 10-12 cm). From each segment sections (6 microm thickness) were immunostained with a pan-neuronal marker (Protein Gene Product 9.5) to visualize the enteric nervous system. A morphometric analysis was carried out for each segment recording the number of ganglia and nerve cells of the submucous plexus. Neither ganglia nor nerve cells showed a uniform distribution pattern, but decreased continuously towards the anus. However, even the lowest segments (S1, S2) contained nerve cells and were not aganglionic. In the remaining segments ganglia with 7 or more nerve cells could be detected. The findings demonstrate segment-specific quantitative differences of the anorectal submucous plexus which should be taken into consideration for the histopathologic evaluation of rectal biopsies. Moreover, the data support the concept of a physiologic hypoganglionosis of the anal canal.

Adult↗

[Supracondylar humerus fracture in childhood--an efficacy study. Results of a multicenter study by the Pediatric Traumatology Section of the German Society of Trauma Surgery--I: Epidemiology, effectiveness evaluation and classification].

In this retrospective study of the pediatric trauma group of German trauma society, issued to investigate the state of the art treatment of the supracondylar fracture of the humerus, 13 clinics took part. In this first part of our study we tested the epidemiology and effectivity of therapeutic interventions based on the classification of v. Laer. 886 fractures were included with an average patients age of 5.8 years (+/- 2.9). Causes of trauma was in 45% playing, followed by school/kindergarden and sports injuries. Fractures were initially classified according to v. Laer and showed following displacement: 35.4% Type I, 21.9% Type II, 18.1% Type III and 24.6% Type IV. 10 of the 886 cases (1.1%) were open fractures. Damages to nerves were described in 45 patients (5.1%) and only 7 (0.7%) had primary vessel lesions. 476 patients were treated by reduction of fragments, 72% using a closed technical approach and 28% using an open approach. 6% underwent a second resposition-maneuver, which was mainly observed after crossed Kirschner-wire in type-III-and-IV-fractures. Therapy was changed in 5.1% mostly of the cases were initially closed reduced and then fixed with a collar and cuff sling. 540 patients were seen at follow-up (61%). 81.1% of these patients showed symmetrical axis compared to the uninjured arm. A varus-deformity was noted in 11.7%, a valgus-deformity in 7.2%. Analysis of effectivity showed that the primarily used classification was not sufficient for prediction of the outcome after reposition and retention. Therefore the classification was modified based on 4 groups: Type I undisplaced, Type II displacement in one plane, Type III displacement in two planes and Type IV displacement in three spatial planes. Using this classification we could found that in group II 25% of reduction an 7% of retentions were ineffective. For group III and IV we found that > 20% of the retention proofed to be ineffective.

Adolescent↗

Parenteral nutrition-induced hepatobiliary dysfunction in infants and prepubertal rabbits.

We analyzed clinical, biochemical, and histo- logic parameters of ten infants with parenteral nutrition-induced hepatobiliary dysfunction. The data were compared with the results of a rabbit model. All infants were born prematurely with low birth weight. Their clinical diagnoses were necrotizing enterocolitis (6), gastroschisis (1), intrauterine volvulus (1), and lung hypoplasia (2). All required total (TPN) or partial parenteral nutrition for at least 8 weeks. All had repeated episodes of infections or sepsis. A rise in bilirubin and aminotransferase levels occurred after a minimum of 5 weeks; peak bilirubin levels ranged from 4 to 14 mg% and aminotransferases from 40 to 140 IU/l. One child later developed gallstones. Liver biopsies after 1 to 24 months showed fibrosis, bile-duct proliferation, cholestasis, and hydropic degeneration. All of the above-mentioned clinical factors have been accused of causing the observed biochemical and histologic changes. In our rabbit model we were able to produce almost identical symptoms by TPN alone: gallbladder distension, sludge, and stones developed after 1-4 weeks of TPN as well as uncharacteristic changes in aminotransferases and bilirubin after 4 weeks. Liver histology revealed severe hydropic degeneration of zone 3 as early as 1 week after beginning TPN. A rise of fibrosis and bile-duct proliferation after 1 to 4 weeks of infusion was statistically significant. Cholestasis, as was observed in the infants, could not be detected. In our model, all alterations observed could be attributed exclusively to TPN. We therefore assume that TPN was the true cause of the dysfunction. In a second experimental series infusions were reduced to 80% PN and free access to lab chow. These animals produced normal feces, indicating physiologic enteral stimulation. They developed the same degenerative and proliferative histologic changes, whereas gallbladder distension, sludge, and stones were not noted. We conclude that: (1) The TPN solution itself is responsible for the histologic changes in the liver, which is supported by the fact that hydropic degeneration of zone 3 is typical of a direct toxic effect; and (2) Complete enteral starvation with an absence of enteral stimulation causes disease of the lower biliary tract.

Animals↗

Could testicular descent in humans be promoted by direct androgen stimulation of the gubernaculum testis?

Gubernaculum testis and androgens play an important role in physiologic testicular descent. A direct androgen stimulation of the gubernaculum is only possible in the presence of specific receptors, which have not yet been demonstrated. We have determined the androgen-binding capacity in 61 samples of gubernaculum testis from 48 patients, 34 samples belonging to patients with maldescended testes. In 43 out of 61 instances we found androgen binding with values up to 72 fmol/mg, with a mean of 13.08 fmol/mg. In 27 samples from patients with a deep scrotal positioned gonad the mean androgen-binding capacity was 18.74 fmol/mg. In 10 samples from patients with a gonad in the proximal scrotum and 19 patients with epifascial ectopy or a low inguinal position the mean androgen-binding capacities decreased progressively to 9 fmol/mg and 5.95 fmol/mg. In the 5 samples from patients with high inguinal or abdominal testes the binding capacity of 17.8 fmol/mg did not significantly differ from the value obtained in the samples of the normal-positioned gonads, suggesting that transabdominal descent is probably not androgen-dependent. We divided the probes into receptor status positive for a binding capacity higher than or equal to 10 fmol/mg, and receptor status negative for a binding capacity under 10 fmol/mg. 63% of the samples from patients with scrotal positioned testes were receptor status positive, 85% of the samples belonging to patients with maldescended testes were receptor status negative. Receptor status was negative in patients with high scrotal, epifascial or low inguinal and high inguinal or abdominal testes in 90%, 95% and 40% of the samples respectively. Immunohistochemical examination of 10 samples showed small cell groups with stained nuclei in 2 out of 5 patients with normal-positioned testes. All other samples were negative. These findings prove that hormone binding was accomplished by specific intranuclear receptors. These findings allow for the first time the hypothesis of a direct androgen stimulation of the human gubernaculum testis. The probable mechanism is an increase in mucopolysaccharides leading to a swelling of the gubernaculum, dilating the inguinal canal and promoting testicular descent. Although certainly a multifactorial process, quality and quantity of androgen receptors would influence the extent of transinguinal testicular descent.

Androgens↗

What do children look like after longitudinal intestinal lengthening.

The longitudinal intestinal lengthening, described by Bianchi in 1980, has been shown to be effective in improving intestinal function, absorption and transit time in patients with short-bowel syndrome. We report the long-term results of 18 survivors of a series of 25 intestinal lengthening procedures performed since 1984. Mean age of the patients was 18 months (range of 5 to 52 months), mean follow-up 6 years (0.9 to 12 years). Parenteral nutrition was progressively reduced in all patients and discontinued after 1 to 10 months (mean 5.1 months). Frequently encountered problems during long-term follow-up are hyperphagia, hyponatremia and hypochloremia, metabolic acidosis, including D-lactic acidosis, cholelithiasis and urolithiasis, gastro-esophageal reflux, dystrophy and symptoms caused by secondary dilatation of the lengthened bowel loops: a protruding abdomen, enteral stasis, leading to constipation or diarrhea with bacterial overgrowth. Overall performance has been acceptable in 13 out of 18 patients. Longitudinal intestinal lengthening is effective enabling patients with short-bowel syndrome to be weaned from parenteral nutrition, allowing for long-term survival. However, it is only one step on a long and difficult way. Multiple problems have to be searched for and adequately dealt with to achieve an acceptable and future worth living.

Anastomosis, Surgical↗

Rectal biopsy for diagnosis of intestinal neuronal dysplasia in children: a prospective multicentre study on interobserver variation and clinical outcome.

BACKGROUND: Intestinal neuronal dysplasia (IND) of the colonic submucous plexus is considered to be a congenital malformation of the enteric nervous system causing symptoms resembling those of Hirschsprung's disease. In contrast with the established diagnosis of aganglionosis using enzyme histochemistry, controversy exists over the diagnostic criteria of IND on rectal biopsies previously defined by a consensus report and the causal relation between morphological findings and clinical symptoms. AIMS: The interobserver variability was prospectively investigated with respect to final diagnoses and several histological features in rectal biopsy specimens from children suspected of having colonic motility disturbances. METHODS: 377 biopsy specimens from 108 children aged 4 days to 15 years were independently coded without knowledge of clinical symptoms by three experienced pathologists for 20 histological features, and a final diagnosis was given for every case. Interobserver variation for the different items and the final diagnosis were analysed using Cohen's kappa statistic. Clinical data at biopsy and outcome after 12 months were related to morphological findings. RESULTS: The three pathologists agreed completely with respect to the diagnosis Hirschsprung's disease (kappa = 1), but in only 14% of the children without aganglionosis. In 15 (17%) of the 87 children without aganglionosis, at least one pathologist judged the case as normal, while another diagnosed IND. kappa values were close to the zero value expected by chance for the diagnoses normal and IND. Young age was related to the presence of several morphological features-for example, acetylcholine esterase staining and presence of giant ganglia. Children with chronic constipation diagnosed as having IND, given no other specific diagnosis by any of the pathologists, were significantly younger (median 8.8 months) and had a higher cure rate after one year (60%) than constipated patients considered by all observers to have no histological abnormalities (median 6.1 years, cure rate 23%). CONCLUSIONS: In contrast with Hirschsprung's disease, there is a high interobserver variation with regard to the different morphological features and final diagnosis of IND, based on the criteria and conditions of the previous consensus report. The high frequency of histological "abnormalities" in young infants suggests that some of the features may represent a normal variant of postnatal development rather than a pathological process. Investigations using more refined and morphometric methods in rectal specimens from infants and children without bowel disease are needed to define the normal range of morphological appearance at different ages. These preliminary data indicate that, with current knowledge, rectal biopsy for diagnostic purposes should only be performed in constipated children for diagnosis of Hirschsprung's disease.

Adolescent↗

Long-term total parenteral nutrition-induced hepatobiliary dysfunction in a rabbit model.

BACKGROUND/PURPOSE: Currently, the reason for hepatobiliary dysfunction associated with long-term total parenteral nutrition (TPN) is much debated and still unclear. No agreement can be achieved about whether bacteriotoxins and sepsis, enteral starvation, consequences of abdominal operations, or the TPN solution itself is the real cause for the disease. Animal models were criticized for their short period of TPN and their failure to demonstrate cholestasis and bile duct proliferation. The aim of this study was to establish an animal model for long-term TPN in which the same alterations of the hepatobiliary system as observed in humans could be produced. METHODS: In this model, rabbits could be kept for the first time under continuous TPN for 4 weeks. Three serial liver biopsy sections were taken operatively from each animal and biochemical analyses were performed four times. A control group of enterally fed rabbits underwent exactly the same procedure in respect to operations and handling, so that differences in macroscopical, biochemical, and histological changes between both groups could be attributed exclusively to TPN. RESULTS: Only in the TPN group gallbladder distension developed in all animals after 1 week. After 3 and 4 weeks, viscous dark bile, sludge and stones, a slight rise in direct bilirubin, and a decline in plasma albumin and alkaline phosphatase was noted. In both groups liver biopsy results showed a similar degree of mild portal inflammation and single-cell necrosis at equivalent time points. These changes could be caused by antiseptics, antibiotics, anesthesia, and operations. Although mild to moderate proliferative changes and no hydropic degeneration developed in the control group during the same time, the TPN group generated marked proliferative and degenerative changes. We noted as early as 1 week after starting TPN a severe hydropic degeneration in 90% of the animals. Fibrosis and bile duct proliferation increased from a slight degree after 1 week up to a moderate to severe degree after 3 and 4 weeks, respectively. CONCLUSIONS: The hepatobiliary alterations associated with TPN in children, which cannot be separated clinically from consequences of multiple other factors, can almost identically be reproduced in our rabbit model as a clear consequence of TPN. Furthermore, the hydropic degeneration of the liver cells begins in zone 3 and is an early predominant feature of hepatobiliary dysfunction in rabbits and infants. It must be rated as a response to a direct cytotoxic effect on the liver cell.

Alanine Transaminase↗

[Pediatric tibial fractures: is conservative therapy still currently appropriate?].

Leg-length inequality is the most common complication reported after pediatric tibial fractures. We reviewed 196 patients with tibial fractures. The mean age at the time of fracture was 7.6 years (range 10 months-12 years). In all, 176 patients were treated conservatively, 162 by immediate cast bracing and 14 by skeletal traction. In 20 patients the fracture was stabilized by osteosynthesis. Thirteen patients were treated by plate fixation; in 4 fractures located in the distal third of the tibia, we used crossed Kirschner wires. In one case we used a screw osteosynthesis; another distal fracture was stabilized by a fibula rushpin, and in one third-degree open fracture an arthrodesis of the upper ankle joint was necessary. A total of 71 patient were interviewed and examined. All were skeletally mature at the time of reexamination. A leg-length discrepancy was found in 40 patients. Shortening of 5-15 mm had occurred in 11 patients; 29 patients had experienced lengthening of 5-20 mm. In 21 patients, leg-length discrepancy was more than 5 mm (13 had lengthening of 10-20 mm, 8 had shortening of 10-15 mm). Leg-length discrepancy was significantly higher in patients younger than 10 years (P = 0.024, chi 2-test according to Pearson). The number of repositions also played an important role (1 or more; P = 0.006). With fibula involvement, leg-length discrepancy was significantly higher (P = 0.018). Both factors indicate the grade of fracture instability. The authors conclude that conservative treatment of pediatric tibial fractures is still appropriate. However, instable fractures as well as fractures in children > 10 years of age should be operated upon primarily.

Casts, Surgical↗

Early wound closure and early reconstruction. Experience with a dermal substitute in a child with 60 per cent surface area burn.

A dermal substitute was used for wound management and after early scar release on a 4-year-old child with mostly full thickness burns covering 60 per cent of the body surface. The biosynthetic material (INTEGRA Artificial Skin) consists of an upper silicone film and a lower layer of porous cross-linked collagen and chondroitin-6-sulfate as a template for dermal regeneration. Eight sheets each 4 x 10 in. were used to cover the patient's whole trunk after staged tangential necrectomy. In the third and fourth weeks following application the silicone layer was easily removed and the newly formed dermis covered with widely meshed, thin split-thickness autograft. Seven weeks after admission an early neck contracture was released and the skin defect also covered with INTEGRA Artificial Skin. Following the same principle, transplantation of the thin unmeshed autograft was performed successfully 3 weeks later. The good results regarding handling, final take, apparent initial scar reduction, and early recovery may favourably effect initial treatment and reconstruction planning after extensive full-thickness burn injuries.

Burns↗

[Value of anorectal manometry in assessment of constipation in childhood].

We studied retrospectively 210 anorectal manometries of constipated children. Of the 87 patients with an anal fissure or a functional constipation, 83 had normal sphincter relaxation. All of the 23 patients with Hirschsprung's disease lacked the sphincter relaxation, as well as 22 of the patients with a dysganglionosis. Eleven patients with innervation defects showed pathologic sphincter contractions. Anorectal manometry is a valuable tool to differentiate between innervation defects and constipation of other etiologies.

Adolescent↗

[Does the degree of dislocation correlate with therapy procedure in supracondylar humerus fractures in childhood?].

Hundred and twenty-eight supracondylar fractures of the humerus were studied retrospectively after an follow-up time of 4.3 years (1 to 17.8 years). In 87 cases (68%) the operative procedure was the closed reduction and percutaneous crossed-pin fixation for 19 fractures type III (56%). 22 fractures type II (76%) and 46 fractures type I (85%), whereas 41 fractures were treated by open reduction and crossed-pin fixation. The findings were evaluated according to "Flynn's criteria" leading to the following results: "excellent" 77 times (60.2%), "good" 44 times (34.4%) "fair" 3 times (2.3%) and "poor" 4 times (3.1%). Our results show that with approximately 50% of all fractures type III the treatment by closed reduction and percutaneous crossed-pin fixation leads to a very good long-term result. On the other hand, rotated or interponated fractures type I and II require an open reduction and crossed-pin fixation. Independent of the type of fracture, the closed reduction and percutaneous crossed-pin fixation should always be taken into consideration. Exceptions are open fractures and those with multiple fragments.

Adolescent↗

[Leg length inequality after childhood femoral fractures--permanent or temporary phenomenon?].

Leg length inequality is the most common complication reported after femoral shaft fractures in childhood. Most authors agree that significant overgrowth occurs in the first two years after injury and will not be further corrected. We reviewed 221 patients (166 boys, 44 girls) with a fracture of the femoral shaft. The mean age at the time of fracture was 6.5 years (range 11 months to 12 years); 123 patients were treated conservatively, 96 by skin traction, 11 by skeletal traction, and 16 by immediate cast bracing. In 98 patients the fracture was stabilized by osteosynthesis. In 5 fractures located in the distal third of the femur we used crossed Kirschner wires. Fifty-nine patients were treated by intramedullary nailing, without problems regarding trochanteric apophyseal arrest or alteration in the collum angle. Thirty-four patients were treated by plate fixation, this being associated with high rates (9%) of implant-breakage. A total of 127 patients were interviewed and examined; they were skeletally mature at the time of reexamination. A leg-length discrepancy was found in 45 patients. Shortening from 10 to 30 mm (mean 14.3 mm) occurred in 7 patients; 38 patients had lengthening from 10 to 25 mm (mean 14.1 mm). Overgrowth significantly depended on the age at trauma (4-9 years; P = 0.04), number of repositions (2 or more; P = 0.0005) and degree of axial deviation (> 10 degrees; P = 0.04). Delayed surgical treatment (> 48 h; P = 0.0035), especially plate fixation (P = 0.0003) induced overgrowth as well. Forty-six patients had previously been reevaluated 12 years before (1981). In 12 patients 13 years or older at the time of the first review, no change in leg-length difference occurred. At the first review 34 patients were younger than 13 years. Eight of them had no leg-length discrepancy. In 16 patients the growth rate of the affected femur decreased, so that leg-length discrepancy diminished after the 2-year period posttraumatically in a range from 5 to 15 mm. Overgrowth of the femur continued in 7 cases ranging from 5 to 10 mm. No change occurred in 3 patients. Thus, there is a further change in length inequality more than 2 years post-traumatically.

Adolescent↗

[Indications and outcome of anal sphincter myectomy in childhood].

Anal sphinctermyectomy according to Lynn is an established therapy for chronic constipation in children. However, no consensus on indications exists and, according to the literature, results are contradictory. In order to clarify this problem, 33 children were examined and divided into two groups: I (n = 12) chronic constipation, and II (n = 21) dysganglionosis. All children were treated conservatively for at least a 6-month period and showed elevated anal resting tone in manometry. The operative results were assessed by anorectal manometry and a physical examination; subjective opinion was evaluated using a questionnaire. Anorectal manometry showed a marked decrease of anal resting tone of 30%, correlating to subjective relief of the symptoms.

Adolescent↗

[Peutz-Jeghers syndrome. Cases at the Mannheim clinic over 25 years].

Observations in our clinic and others reviewed in the literature result in a new picture of the Peutz-Jeghers syndrome. It turns out to be a hereditary polypose syndrome which is hard to define. The tendency to malignant degeneration of polyps and development of associated neoplasms is almost impossible to forecast. In addition operations frequently involve complications and often need to be repeated more than once. Radical removal of all polyps must be the aim of treatment; the patients must then be closely followed up by clinical examination, endoscopy and radiology to avoid complications of regrowth and to make sure degeneration and associated neoplasms are detected at an early stage.

Adolescent↗

[Overflow incontinence--a rare manifestation of Peutz-Jeghers syndrome].

We present the case of a 12-year-old girl with Peutz-Jeghers syndrome (PJS). Enormous polyps of the transverse colon intussuscepting into the rectum turned out to be the rate cause of overflow incontinence. Although she presented all the signs of PJS at infancy, diagnosis was made only after a period of nine years, implying a lack of follow-up. We emphasize that when polyps occur in children, a complete examination is required, and once the diagnosis of Peutz-Jeghers syndrome is established, frequent endoscopic follow-up and resection of polyps are necessary.

Adenomatous Polyposis Coli↗